Myeloid neoplasm with histiocytosis and spleen tyrosine kinase fusion responds to fostamatinib

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Society of Dermatology Hospitalists supportive care guidelines for the management of Stevens-Johnson syndrome/toxic epidermal necrolysis in adults

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are life-threatening conditions with high morbidity and mortality. Supportive care management of SJS/TEN is highly variable. A systematic review of the literature was performed by dermatologists, ophthalmologists, intensivists, and gynecologists with expertise in SJS/TEN to generate statements for supportive care guideline development. Members of the Society of Dermatology Hospitalists with expertise in SJS/TEN were invited to participate in a modified, online Delphi-consensus. Participants were administered 9-point Likert scale questionnaires regarding 135 statements. The RAND/UCLA Appropriateness Method was used to evaluate and select proposed statements for guideline inclusion; statements with median ratings of 6.5 to 9 and a disagreement index of ≤1 were included in the guideline. For the final round, the guidelines were appraised by all of the participants. Included are an evidence-based discussion and recommendations for hospital setting and care team, wound care, ocular care, oral care, urogenital care, pain management, infection surveillance, fluid and electrolyte management, nutrition and stress ulcer prophylaxis, airway management, and anticoagulation in adult patients with SJS/TEN

Updates in SJS/TEN: collaboration, innovation, and community

Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis (SJS/TEN) is a predominantly drug-induced disease, with a mortality rate of 15–20%, that engages the expertise of multiple disciplines: dermatology, allergy, immunology, clinical pharmacology, burn surgery, ophthalmology, urogynecology, and psychiatry. SJS/TEN has an incidence of 1–5/million persons per year in the United States, with even higher rates globally. One of the challenges of SJS/TEN has been developing the research infrastructure and coordination to answer questions capable of transforming clinical care and leading to improved patient outcomes. SJS/TEN 2021, the third research meeting of its kind, was held as a virtual meeting on August 28–29, 2021. The meeting brought together 428 international scientists, in addition to a community of 140 SJS/TEN survivors and family members. The goal of the meeting was to brainstorm strategies to support the continued growth of an international SJS/TEN research network, bridging science and the community. The community workshop section of the meeting focused on eight primary themes: mental health, eye care, SJS/TEN in children, non-drug induced SJS/TEN, long-term health complications, new advances in mechanisms and basic science, managing long-term scarring, considerations for skin of color, and COVID-19 vaccines. The meeting featured several important updates and identified areas of unmet research and clinical need that will be highlighted in this white paper

Acitretin amelioration of Acrokeratosis Paraneoplastica (Bazex Syndrome) in cases of incurable squamous cell carcinoma of the hypopharynx

BACKGROUNDAcrokeratosis paraneoplastica (Bazex Syndrome) is a rare paraneoplastic syndrome and dermatosis that only arises in patients with underlying malignancy and uncommonly resolves with systemic therapy.OBJECTIVE/METHODSWe present a patient with acrokeratosis paraneoplastica that improved significantly with acitretin. We present evidence to justify costs of therapy for insurance purposes. Additionally, there is a single report of acitretin use for Bazex syndrome in the French language.RESULTSWe present a case of acrokeratosis paraneoplastica in a patient with incurable stage IV squamous cell carcinoma of the hypopharynx that significantly improved on acitretin.CONCLUSIONAlthough acrokeratosis paraneoplastica most often is cured by treatment of the underlying squamous cell carcinoma, this case highlights the potential benefit of early initiation of acitretin during malignancy work up and staging. This therapy may also be valuable for patients in which the primary malignancy is unresectable or incurable

Scoring Assessments in Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis

Epidermal necrolysis, the unifying term for Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN), is a severe cutaneous drug reaction associated with high morbidity and mortality. Given the rarity of this disease, large-scale prospective research studies are limited. Significant institutional and geographical variations in treatment practices highlight the need for standardization of clinical assessment scores and prioritization of research outcome measures in epidermal necrolysis. At the present, clinical assessment is typically simplified to total body surface area (BSA) involvement, with little focus on morphology. Validated clinical scoring systems are used as mortality prognostication tools, with SCORTEN being the best-validated tool thus far, although the ABCD-10 has also been recently introduced. These tools are imperfect in that they tend to either overestimate or underestimate mortality in certain populations and are not designed to monitor disease progression. Although mortality is often used as a primary endpoint for epidermal necrolysis studies, this outcome fails to capture more nuanced changes in skin disease such as arrest of disease progression while also lacking a validated skin-directed inclusion criterion to stratify patients based on the severity of skin disease at study entry. In addition to mortality, many studies also use BSA stabilization or time to re-epithelialization as endpoints, although these are not clearly defined morphologically, and inter- and intra-rater reliability are unclear. More specific, validated cutaneous assessment scores are necessary in order advance therapeutic options for epidermal necrolysis. In this review, we summarize the strengths and weaknesses of current clinical assessment practices in epidermal necrolysis and highlight the need for standardized research tools to monitor cutaneous involvement throughout the hospitalization

Pasteurella multocida ecthyma complicated by necrotizing fasciitis

Necrotizing fasciitis is a serious infection of theskin and soft tissues. Pasteurella multocida israrely reported to cause necrotizing fasciitis and isassociated with high mortality. We describe a femalepatient with a past medical history of diabetesmellitus and myeloproliferative disorder presentingwith bullae and erythema of the right forearmsecondary to P. multocida infection after possiblecat bite. Despite adequate antibiotic coverage shedeveloped necrotizing fasciitis diagnosed clinicallyand on diagnostic imaging. Patient was taken to theoperating room emergently and underwent irrigationand debridement with subsequent split-skin graft. Sherecovered well after the surgeries and was dischargeon intravenous antibiotics. At clinic follow-up, herwounds were healing well without any significantnew symptoms

Temozolomide-induced inflammation of disseminated superficial actinic porokeratosis

We report a case of temozolomide (TMZ)-induced inflammation of disseminated superficial actinic porokeratosis (DSAP), an uncommon and pre-malignant cutaneous disorder. Dermatologists and oncologists should be aware of this cutaneous eruption of DSAP associated with TMZ to prevent the discontinuation of effective medical therapy in cancer patients